EHA and TIF explore how COVID-19 is affecting thalassemia and SCD patients

In a webinar designed to guide physicians in the care of hematology patients during the COVID-19 pandemic, three world experts on thalassemia and sickle cell disease (SCD) provided on-the-ground information from physicians who were dealing with the height of the crisis in their countries.

The webinar was organized by the European Hematology Association (EHA) and the Thalassemia International Federation (TIF).

Italian experience with thalassemia and COVID-19

Dr. Cappellini discussed the Italian experience with 11 thalassemia patients followed by a network survey who developed COVID-19 in the northern part of Italy, where the pandemic has been most widespread.

There are no published data focusing specifically on SARS-CoV-2 infection in patients with thalassemic syndromes, but patients with preexisting comorbidities are likely to be more severely affected by SARS-CoV-2, according to Dr. Cappellini.

Of particular concern is the fact that patients with thalassemia, especially older ones, are frequently splenectomized, which renders them more vulnerable to bacterial infections and can trigger life-threatening sepsis. However, splenectomy is not known to increase the risk of viral infection or severe viral illness. Of additional concern is the fact that many thalassemia patients need routine and frequent transfusions.

Overall, the 11 thalassemia patients who developed COVID-19 experienced only mild to moderate symptoms. This is despite the fact that 72% of the patients were splenectomized, which did not appear to affect the clinical course, and all of the patients had thalassemia-related comorbidities.

Around half of the patients were hospitalized, but none of them required transfer to the ICU. One patient who was treated with chemotherapy for diffuse large B-cell lymphoma in 2019 but is now in remission required more intense ventilation support with the use of continuous positive airway pressure.

Only three patients received specific treatment for COVID-19: one with hydroxychloroquine (HCQ) alone, one with HCQ plus anakinra, and one with HCQ plus ritonavir/darunavir.

Overall, “the number of infected thalassemia patients was lower than expected, likely due to earlier and more vigilant self-isolation compared to the general population,” Dr. Cappellini said. She pointed out that the first early response in February by thalassemia physicians was to warn their patients via email and phone calls about the need for self-isolation and precautions against the pandemic.

Results in additional thalassemia patients and SCD patients

Dr. Eleftheriou described 51 cases of thalassemia patients with SARS-CoV-2 infection reported to TIF as of April 16. Patients were from Cyprus, Italy, the United Kingdom, France, Turkey, Iran, Pakistan, and Indonesia.

Of the 51 patients, 46 presented with mild to moderate symptoms. Five patients had severe respiratory symptoms and required hospitalization, two were hospitalized and discharged, and three died between day 5 and day 15 post hospitalization.

All 32 SCD patients were screened and treated for COVID-19, and 17 of them continued treatment for 10 days. In all, 22 patients were hospitalized, 11 were transferred to the ICU, and 1 died.

Ensuring adequate blood supply

Dr. Eleftheriou also discussed the TIF response to the COVID-19 pandemic, which focused on the adequacy of blood supplies for these patients who so often need transfusions.

Dr. Eleftheriou stated that a shortage of blood was reported in 75% of the 62 member countries of the TIF, with 58% reporting severe shortages and 35% reporting moderate to severe shortages.

The shortages resulted in many countries returning to older family/friends donation practices, rare use of whole blood transfusions, and the use of older blood transfusions (older than 28 days).

In addition, physicians have modified their transfusion strategy. They have reduced the amount of blood given to thalassemia patients from two units to one unit during any transfusion, while making arrangements for more frequent transfusions; for example, one transfusion per week but with precautions made to “limit the time spent in the clinic and to control blood supplies while safeguarding that all [thalassemia] patients will be able to get their transfusion,” Dr. Eleftheriou said.

The information in the webinar was provided with the caveat that “no general evidence-based guidance can be derived from this discussion.” There were no other disclosures given.